Pulmonary hypertension in connective tissue diseases

Objective To determine the incidence and severity of pulmonary hypertension (PHT) secondary to connective tissue diseases (CTD).Methods A retrospective study was performed in this hospital on 700 inpatients with different kinds of CTD including systemic lupus erythematosus (SLE),systemic sclerosis (SSc),polymyositis (PM),dermatomyositis (DM),primary Sjgren′s syndrome (pSS) and mixed connective tissue diseases (MCTD).Results PHT occurred in 11 of 81 SSc (13 6%),10 of 164 DM/PM (6 1%),6 of 116 pSS (5 2%),3 of 62 MCTD (4 8%) and 10 of 277 SLE (3 6%) respectively.The incidence rate of severe PHT was 72 2% in SSc,40 0% in SLE,33 3% in pSS and MCTD,20 0% in DM/PM;PHT secondary to DM/PM,pSS and SLE statistically correlated with interstitial lung disease while correlation with Raynaud′s phenomenon only existed in PHT secondary to SSc、pSS and SLE.The sensitivity of Doppler echocardiography in detecting PHT was 83 3%,much higher than that of electrocardiography and chest radiography.Conclusion It seems that the pathogenesis and severity of PHT in different CTD are not the same.Understanding the mechanisms of PHT in CTD will serve to aid the design of rational approaches to reverse the disease.Doppler echocardiography is the most effective noninvasive method in detecting PHT.